Pulmonary changes in the index patient. A: Diffuse bilateral linear and reticular opacities, compatible with interstitial pulmonary involvement (chest X-ray). B: Diffuse subpleural fibrotic changes – honeycomb (black asterisk), areas of subpleural consolidations (blue asterisk) and bronchectasis (red asterisk) in the upper lobes (high-resolution computer tomography scan). C: Chronic reparative and resorptive reaction: fibrosis and cystic rearrangement (green arrows) and cholesterol clefts (blue arrows) in the upper left lobe (hematoxylin and eosin tissue stain; original magnification 50x). D: Thickened arterial wall, destruction of the elastic layer, thrombosis showing organizing vasculitis (red arrows) in the upper left lobe (elastin tissue stain; original magnification 250x).